BRANCHED-CHAIN AMINO-ACID FREE PARENTERAL-NUTRITION IN THE TREATMENT OF ACUTE METABOLIC DECOMPENSATION IN PATIENTS WITH MAPLE SYRUP URINE DISEASE

被引：26

作者：

BERRY, GT ^{[1
]}

HEIDENREICH, R ^{[1
]}

KAPLAN, P ^{[1
]}

LEVINE, F ^{[1
]}

MAZUR, A ^{[1
]}

PALMIERI, MJ ^{[1
]}

YUDKOFF, M ^{[1
]}

SEGAL, S ^{[1
]}

机构：

[1] UNIV PENN,SCH MED,DEPT PEDIAT & MED,PHILADELPHIA,PA 19104

来源：

NEW ENGLAND JOURNAL OF MEDICINE | 1991年 / 324卷 / 03期

关键词：

D O I：

10.1056/NEJM199101173240307

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

MAPLE syrup urine disease is a rare inborn error of branched-chain amino acid and branched-chain keto acid metabolism due to decreased branched-chain α-keto acid dehydrogenase-complex activity.1 The branched-chain keto acids, which are the substrates in this irreversible enzyme reaction, are derived from transamination of the branched-chain amino acids leucine, isoleucine, and valine. Consumption of branched-chain amino acids in the form of dietary protein in excess of an age-dependent daily requirement for protein synthesis results in the accumulation of branched-chain amino acids and branched-chain keto acids in the tissues and body fluids of patients in whom there is little or no. . . © 1991, Massachusetts Medical Society. All rights reserved.

引用

页码：175 / 179

页数：5

共 7 条

[1]

[Anonymous], 1989, RECOMMENDED DIETARY

[2]

Danner DJ, 1989, METABOLIC BASIS INHE, P671

[3]

ELSAS LJ, 1988, MODERN NUTRITION HLT, P1337

[4] CLINICAL AND LABORATORY OBSERVATIONS - NEONATAL HEMODIALYSIS - EFFECTIVE THERAPY FOR THE ENCEPHALOPATHY OF INBORN-ERRORS OF METABOLISM [J].

RUTLEDGE, SL ;

HAVENS, PL ;

HAYMOND, MW ;

MCLEAN, RH ;

KAN, JS ;

BRUSILOW, SW .

JOURNAL OF PEDIATRICS, 1990, 116 (01) :125-128

[5] TOTAL PARENTERAL-NUTRITION THERAPY OF TOXIC MAPLE SYRUP URINE DISEASE [J].

TOWNSEND, I ;

KERR, DS .

AMERICAN JOURNAL OF CLINICAL NUTRITION, 1982, 36 (02) :359-365

[6] INSULIN TO ASSIST TREATMENT OF ACUTE EPISODES IN MAPLE SYRUP URINE DISEASE [J].

WENDEL, U ;

LOMBECK, I ;

BREMER, HJ ;

LANGENBECK, U .

JOURNAL OF INHERITED METABOLIC DISEASE, 1982, 5 :25-26

[7]

1985, WHO TECH REP SER, V724, P71

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